A reassessment of the International Study Group criteria for the diagnosis (classification) of Behçet's syndrome.

OBJECTIVE: Patients with ulcerative colitis (UC) and Crohn's disease (CD) were not represented in the diseased controls group that had been utilised in the development of the International Study Group (ISG) criteria for the diagnosis of Behçet's syndrome (BS). Having similar features, both of these conditions can pose problems in the differential diagnosis of BS. Moreover, there has been a recent awareness of coexistence of BS and familial Mediterranean fever (FMF). The aim of this study was to reassess the performance of ISG criteria among patients with BS and other rheumatological conditions, specifically including those with CD, UC, and FMF. METHODS: 302 consecutive patients with BS and 438 patients with other rheumatological conditions were surveyed for the presence or absence of the features of BS by means of a standard form which had been prepared according to ISG criteria. All control patients with a history of oral ulcer had a pathergy test and an eye examination by an experienced ophthalmologist with a slit lamp. The sensitivity and specificity of the ISG criteria were calculated. RESULTS: Seven of 302 patients with BS (2%) did not fulfill the ISG criteria while 5 of 438 controls (1%) fulfilled the ISG criteria. CONCLUSION: In this study ISG criteria performed well in correctly classifying BS. Further specificity studies might be considered in CD.

Juvenile rheumatoid arthritis and common variable hypogammaglobulinemia.

We describe a 9-year-old white boy with systemic juvenile rheumatoid arthritis (JRA) who developed pancytopenia and hypersplenism at the age of 13 years. He underwent splenectomy and 3 years later he developed Coombs' positive hemolytic anemia, alopecia, juvenile warts, and multiple bacterial infections. At that time, investigations were compatible with severe hypogammaglobulinemia associated with common variable immunodeficiency. Concomitantly with this condition he experienced complete remission of his inflammatory arthritis. Immunologic studies of B and T lymphocyte function showed that the number of circulating T and B lymphocytes were normal, while T cell function was depressed, as evidenced by markedly reduced proliferative responses to mitogens and antigens, and ability to mediate B cell help. In addition, his circulating B cells were unable to secrete IgM or IgG. He also exhibited anergy to intradermal challenge with a battery of common antigens. The literature dealing with this clinical association is reviewed, and possible immunologic mechanisms involved are discussed.

AA-amyloidosis presenting with chronic diarrhea and cardiac manifestations.

In secondary amyloidosis (AA type), clinically significant cardiac and gastrointestinal involvement are uncommon, in contrast to the primary type. We report a case presenting with chronic diarrhea and cardiac manifestations who was diagnosed as having AA-amyloidosis with unknown predisposing illness based on endomyocardial, rectal and subcutaneous fat tissue biopsies.